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Journal of the Royal Society of Medicine logoLink to Journal of the Royal Society of Medicine
. 1998;91(Suppl 34):2–13. doi: 10.1177/014107689809134s02

Uses and abuses of enzyme therapy in cystic fibrosis.

P Durie 1, D Kalnins 1, L Ellis 1
PMCID: PMC1296367  PMID: 9709382

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Selected References

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  1. Amarri S., Harding M., Coward W. A., Evans T. J., Weaver L. T. 13Carbon mixed triglyceride breath test and pancreatic enzyme supplementation in cystic fibrosis. Arch Dis Child. 1997 Apr;76(4):349–351. doi: 10.1136/adc.76.4.349. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Beverley D. W., Kelleher J., MacDonald A., Littlewood J. M., Robinson T., Walters M. P. Comparison of four pancreatic extracts in cystic fibrosis. Arch Dis Child. 1987 Jun;62(6):564–568. doi: 10.1136/adc.62.6.564. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Borowitz D. S., Grand R. J., Durie P. R. Use of pancreatic enzyme supplements for patients with cystic fibrosis in the context of fibrosing colonopathy. Consensus Committee. J Pediatr. 1995 Nov;127(5):681–684. doi: 10.1016/s0022-3476(95)70153-2. [DOI] [PubMed] [Google Scholar]
  4. Boyle B. J., Long W. B., Balistreri W. F., Widzer S. J., Huang N. Effect of cimetidine and pancreatic enzymes on serum and fecal bile acids and fat absorption in cystic fibrosis. Gastroenterology. 1980 May;78(5 Pt 1):950–953. [PubMed] [Google Scholar]
  5. Brady M. S., Rickard K., Yu P. L., Eigen H. Effectiveness and safety of small vs. large doses of enteric coated pancreatic enzymes in reducing steatorrhea in children with cystic fibrosis: a prospective randomized study. Pediatr Pulmonol. 1991;10(2):79–85. doi: 10.1002/ppul.1950100208. [DOI] [PubMed] [Google Scholar]
  6. Cleghorn G., Benjamin L., Corey M., Forstner G., Dati F., Durie P. Serum immunoreactive pancreatic lipase and cationic trypsinogen for the assessment of exocrine pancreatic function in older patients with cystic fibrosis. Pediatrics. 1986 Mar;77(3):301–306. [PubMed] [Google Scholar]
  7. Colombo C., Maiavacca R., Ronchi M., Consalvo E., Amoretti M., Giunta A. The steatocrit: a simple method for monitoring fat malabsorption in patients with cystic fibrosis. J Pediatr Gastroenterol Nutr. 1987 Nov-Dec;6(6):926–930. [PubMed] [Google Scholar]
  8. Couper R. T., Corey M., Durie P. R., Forstner G. G., Moore D. J. Longitudinal evaluation of serum trypsinogen measurement in pancreatic-insufficient and pancreatic-sufficient patients with cystic fibrosis. J Pediatr. 1995 Sep;127(3):408–413. doi: 10.1016/s0022-3476(95)70072-2. [DOI] [PubMed] [Google Scholar]
  9. Cox K. L., Isenberg J. N., Osher A. B., Dooley R. R. The effect of cimetidine on maldigestion in cystic fibrosis. J Pediatr. 1979 Mar;94(3):488–492. doi: 10.1016/s0022-3476(79)80609-5. [DOI] [PubMed] [Google Scholar]
  10. Davidson G. P., Hassel F. M., Crozier D., Corey M., Forstner G. G. Iatrogenic hyperuricemia in children with cystic fibrosis. J Pediatr. 1978 Dec;93(6):976–978. doi: 10.1016/s0022-3476(78)81225-6. [DOI] [PubMed] [Google Scholar]
  11. DiMagno E. P. Controversies in the treatment of exocrine pancreatic insufficiency. Dig Dis Sci. 1982 Jun;27(6):481–484. doi: 10.1007/BF01296724. [DOI] [PubMed] [Google Scholar]
  12. DiMagno E. P., Go V. L., Summerskill W. H. Relations between pancreatic enzyme outputs and malabsorption in severe pancreatic insufficiency. N Engl J Med. 1973 Apr 19;288(16):813–815. doi: 10.1056/NEJM197304192881603. [DOI] [PubMed] [Google Scholar]
  13. DiMagno E. P., Malagelada J. R., Go V. L., Moertel C. G. Fate of orally ingested enzymes in pancreatic insufficiency. Comparison of two dosage schedules. N Engl J Med. 1977 Jun 9;296(23):1318–1322. doi: 10.1056/NEJM197706092962304. [DOI] [PubMed] [Google Scholar]
  14. Durie P. R., Bell L., Linton W., Corey M. L., Forstner G. G. Effect of cimetidine and sodium bicarbonate on pancreatic replacement therapy in cystic fibrosis. Gut. 1980 Sep;21(9):778–786. doi: 10.1136/gut.21.9.778. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Durie P. R., Forstner G. G., Gaskin K. J., Moore D. J., Cleghorn G. J., Wong S. S., Corey M. L. Age-related alterations of immunoreactive pancreatic cationic trypsinogen in sera from cystic fibrosis patients with and without pancreatic insufficiency. Pediatr Res. 1986 Mar;20(3):209–213. doi: 10.1203/00006450-198603000-00002. [DOI] [PubMed] [Google Scholar]
  16. Dutta S. K., Hubbard V. S., Appler M. Critical examination of therapeutic efficacy of a pH-sensitive enteric-coated pancreatic enzyme preparation in treatment of exocrine pancreatic insufficiency secondary to cystic fibrosis. Dig Dis Sci. 1988 Oct;33(10):1237–1244. doi: 10.1007/BF01536673. [DOI] [PubMed] [Google Scholar]
  17. Dyck W. P. Titrimetric measurements of fecal trypsin and chymotrypsin in cystic fibrosis with pancreatic exocrine insufficiency. Am J Dig Dis. 1967 Mar;12(3):310–317. doi: 10.1007/BF02233651. [DOI] [PubMed] [Google Scholar]
  18. FitzSimmons S. C., Burkhart G. A., Borowitz D., Grand R. J., Hammerstrom T., Durie P. R., Lloyd-Still J. D., Lowenfels A. B. High-dose pancreatic-enzyme supplements and fibrosing colonopathy in children with cystic fibrosis. N Engl J Med. 1997 May 1;336(18):1283–1289. doi: 10.1056/NEJM199705013361803. [DOI] [PubMed] [Google Scholar]
  19. Gan K. H., Heijerman H. G., Geus W. P., Bakker W., Lamers C. B. Comparison of a high lipase pancreatic enzyme extract with a regular pancreatin preparation in adult cystic fibrosis patients. Aliment Pharmacol Ther. 1994 Dec;8(6):603–607. doi: 10.1111/j.1365-2036.1994.tb00337.x. [DOI] [PubMed] [Google Scholar]
  20. Gaskin K. J., Durie P. R., Lee L., Hill R., Forstner G. G. Colipase and lipase secretion in childhood-onset pancreatic insufficiency. Delineation of patients with steatorrhea secondary to relative colipase deficiency. Gastroenterology. 1984 Jan;86(1):1–7. [PubMed] [Google Scholar]
  21. Gow R., Bradbear R., Francis P., Shepherd R. Comparative study of varying regimens to improve steatorrhoea and creatorrhoea in cystic fibrosis: Effectiveness of an enteric-coated preparation with and without antacids and cimetidine. Lancet. 1981 Nov 14;2(8255):1071–1074. doi: 10.1016/s0140-6736(81)91276-9. [DOI] [PubMed] [Google Scholar]
  22. Graham D. Y. Enzyme replacement therapy of exocrine pancreatic insufficiency in man. Relations between in vitro enzyme activities and in vivo potency in commercial pancreatic extracts. N Engl J Med. 1977 Jun 9;296(23):1314–1317. doi: 10.1056/NEJM197706092962303. [DOI] [PubMed] [Google Scholar]
  23. Graham D. Y. Pancreatic enzyme replacement: the effect of antacids or cimetidine. Dig Dis Sci. 1982 Jun;27(6):485–490. doi: 10.1007/BF01296725. [DOI] [PubMed] [Google Scholar]
  24. Harper T. B., 3rd Relationship of nutrition and pulmonary function in cystic fibrosis. J Pediatr. 1983 Jul;103(1):164–165. doi: 10.1016/s0022-3476(83)80807-5. [DOI] [PubMed] [Google Scholar]
  25. Hiele M., Ghoos Y., Rutgeerts P., Vantrappen G. Starch digestion in normal subjects and patients with pancreatic disease, using a 13CO2 breath test. Gastroenterology. 1989 Feb;96(2 Pt 1):503–509. doi: 10.1016/0016-5085(89)91577-1. [DOI] [PubMed] [Google Scholar]
  26. Kerem E., Corey M., Kerem B. S., Rommens J., Markiewicz D., Levison H., Tsui L. C., Durie P. The relation between genotype and phenotype in cystic fibrosis--analysis of the most common mutation (delta F508). N Engl J Med. 1990 Nov 29;323(22):1517–1522. doi: 10.1056/NEJM199011293232203. [DOI] [PubMed] [Google Scholar]
  27. Khouri M. R., Huang G., Shiau Y. F. Sudan stain of fecal fat: new insight into an old test. Gastroenterology. 1989 Feb;96(2 Pt 1):421–427. doi: 10.1016/0016-5085(89)91566-7. [DOI] [PubMed] [Google Scholar]
  28. Kopelman H., Corey M., Gaskin K., Durie P., Weizman Z., Forstner G. Impaired chloride secretion, as well as bicarbonate secretion, underlies the fluid secretory defect in the cystic fibrosis pancreas. Gastroenterology. 1988 Aug;95(2):349–355. doi: 10.1016/0016-5085(88)90490-8. [DOI] [PubMed] [Google Scholar]
  29. Kraisinger M., Hochhaus G., Stecenko A., Bowser E., Hendeles L. Clinical pharmacology of pancreatic enzymes in patients with cystic fibrosis and in vitro performance of microencapsulated formulations. J Clin Pharmacol. 1994 Feb;34(2):158–166. doi: 10.1002/j.1552-4604.1994.tb03981.x. [DOI] [PubMed] [Google Scholar]
  30. Kristidis P., Bozon D., Corey M., Markiewicz D., Rommens J., Tsui L. C., Durie P. Genetic determination of exocrine pancreatic function in cystic fibrosis. Am J Hum Genet. 1992 Jun;50(6):1178–1184. [PMC free article] [PubMed] [Google Scholar]
  31. LUNDH G. Pancreatic exocrine function in neoplastic and inflammatory disease; a simple and reliable new test. Gastroenterology. 1962 Mar;42:275–280. [PubMed] [Google Scholar]
  32. Lancellotti L., Cabrini G., Zanolla L., Mastella G. High- versus low-lipase acid-resistant enzyme preparations in cystic fibrosis: a crossover randomized clinical trial. J Pediatr Gastroenterol Nutr. 1996 Jan;22(1):73–78. doi: 10.1097/00005176-199601000-00012. [DOI] [PubMed] [Google Scholar]
  33. Laufer D., Cleghorn G., Forstner G., Ellis L., Koren G., Durie P. The bentiromide test using plasma p-aminobenzoic acid for diagnosing pancreatic insufficiency in young children. The effect of two different doses and a liquid meal. Gastroenterology. 1991 Jul;101(1):207–213. doi: 10.1016/0016-5085(91)90479-5. [DOI] [PubMed] [Google Scholar]
  34. Layer P., Go V. L., DiMagno E. P. Fate of pancreatic enzymes during small intestinal aboral transit in humans. Am J Physiol. 1986 Oct;251(4 Pt 1):G475–G480. doi: 10.1152/ajpgi.1986.251.4.G475. [DOI] [PubMed] [Google Scholar]
  35. Meyer J. H., Elashoff J., Porter-Fink V., Dressman J., Amidon G. L. Human postprandial gastric emptying of 1-3-millimeter spheres. Gastroenterology. 1988 Jun;94(6):1315–1325. doi: 10.1016/0016-5085(88)90669-5. [DOI] [PubMed] [Google Scholar]
  36. Murphy J. L., Wootton S. A., Bond S. A., Jackson A. A. Energy content of stools in normal healthy controls and patients with cystic fibrosis. Arch Dis Child. 1991 Apr;66(4):495–500. doi: 10.1136/adc.66.4.495. [DOI] [PMC free article] [PubMed] [Google Scholar]
  37. O'Hare M. M., McMaster C., Dodge J. A. Stated versus actual lipase activity in pancreatic enzyme supplements: implications for clinical use. J Pediatr Gastroenterol Nutr. 1995 Jul;21(1):59–63. doi: 10.1097/00005176-199507000-00010. [DOI] [PubMed] [Google Scholar]
  38. Remtulla M. A., Durie P. R., Goldberg D. M. Is chymotrypsin output a better diagnostic index than the measurement of chymotrypsin in random stool? Enzyme. 1988;39(4):190–198. doi: 10.1159/000469118. [DOI] [PubMed] [Google Scholar]
  39. Robinson P. J., Olinsky A., Smith A. L., Chitravanshi S. B. High compared with standard dose lipase pancreatic supplement. Arch Dis Child. 1989 Jan;64(1):143–145. doi: 10.1136/adc.64.1.143. [DOI] [PMC free article] [PubMed] [Google Scholar]
  40. Robinson P. J., Smith A. L., Sly P. D. Duodenal pH in cystic fibrosis and its relationship to fat malabsorption. Dig Dis Sci. 1990 Oct;35(10):1299–1304. doi: 10.1007/BF01536423. [DOI] [PubMed] [Google Scholar]
  41. Schöni M., Kraemer R., Ruedeberg A., Lentze M. J., Mordasini R. C., Riesen W. F., Kläy M. P., Rossi E. Long-term cimetidine in children with cystic fibrosis: a randomized double-blind study. Pediatr Res. 1984 Jan;18(1):66–70. [PubMed] [Google Scholar]
  42. Smyth R. L., Ashby D., O'Hea U., Burrows E., Lewis P., van Velzen D., Dodge J. A. Fibrosing colonopathy in cystic fibrosis: results of a case-control study. Lancet. 1995 Nov 11;346(8985):1247–1251. doi: 10.1016/s0140-6736(95)91860-4. [DOI] [PubMed] [Google Scholar]
  43. Smyth R. L., van Velzen D., Smyth A. R., Lloyd D. A., Heaf D. P. Strictures of ascending colon in cystic fibrosis and high-strength pancreatic enzymes. Lancet. 1994 Jan 8;343(8889):85–86. doi: 10.1016/s0140-6736(94)90817-6. [DOI] [PubMed] [Google Scholar]
  44. Soldan W., Henker J., Sprössig C. Sensitivity and specificity of quantitative determination of pancreatic elastase 1 in feces of children. J Pediatr Gastroenterol Nutr. 1997 Jan;24(1):53–55. doi: 10.1097/00005176-199701000-00013. [DOI] [PubMed] [Google Scholar]
  45. Stapleton F. B., Kennedy J., Nousia-Arvanitakis S., Linshaw M. A. Hyperuricosuria due to high-dose pancreatic extract therapy in cystic fibrosis. N Engl J Med. 1976 Jul 29;295(5):246–248. doi: 10.1056/NEJM197607292950503. [DOI] [PubMed] [Google Scholar]
  46. Stead R. J., Skypala I., Hodson M. E., Batten J. C. Enteric coated microspheres of pancreatin in the treatment of cystic fibrosis: comparison with a standard enteric coated preparation. Thorax. 1987 Jul;42(7):533–537. doi: 10.1136/thx.42.7.533. [DOI] [PMC free article] [PubMed] [Google Scholar]
  47. Stein J., Jung M., Sziegoleit A., Zeuzem S., Caspary W. F., Lembcke B. Immunoreactive elastase I: clinical evaluation of a new noninvasive test of pancreatic function. Clin Chem. 1996 Feb;42(2):222–226. [PubMed] [Google Scholar]
  48. Thiruvengadam R., DiMagno E. P. Inactivation of human lipase by proteases. Am J Physiol. 1988 Oct;255(4 Pt 1):G476–G481. doi: 10.1152/ajpgi.1988.255.4.G476. [DOI] [PubMed] [Google Scholar]
  49. Toskes P. P. Bentiromide as a test of exocrine pancreatic function in adult patients with pancreatic exocrine insufficiency. Determination of appropriate dose and urinary collection interval. Gastroenterology. 1983 Sep;85(3):565–569. [PubMed] [Google Scholar]
  50. Twarog F. J., Weinstein S. F., Khaw K. T., Strieder D. J., Colten H. R. Hypersensitivity to pancreatic extracts in parents of patients with cystic fibrosis. J Allergy Clin Immunol. 1977 Jan;59(1):35–40. doi: 10.1016/0091-6749(77)90174-9. [DOI] [PubMed] [Google Scholar]
  51. Waters D. L., Dorney S. F., Gaskin K. J., Gruca M. A., O'Halloran M., Wilcken B. Pancreatic function in infants identified as having cystic fibrosis in a neonatal screening program. N Engl J Med. 1990 Feb 1;322(5):303–308. doi: 10.1056/NEJM199002013220505. [DOI] [PubMed] [Google Scholar]
  52. Weizman Z., Forstner G. G., Gaskin K. J., Kopelman H., Wong S., Durie P. R. Bentiromide test for assessing pancreatic dysfunction using analysis of para-aminobenzoic acid in plasma and urine. Studies in cystic fibrosis and Shwachman's syndrome. Gastroenterology. 1985 Sep;89(3):596–604. doi: 10.1016/0016-5085(85)90456-1. [DOI] [PubMed] [Google Scholar]
  53. Youngberg C. A., Berardi R. R., Howatt W. F., Hyneck M. L., Amidon G. L., Meyer J. H., Dressman J. B. Comparison of gastrointestinal pH in cystic fibrosis and healthy subjects. Dig Dis Sci. 1987 May;32(5):472–480. doi: 10.1007/BF01296029. [DOI] [PubMed] [Google Scholar]
  54. Zentler-Munro P. L., Fine D. R., Batten J. C., Northfield T. C. Effect of cimetidine on enzyme inactivation, bile acid precipitation, and lipid solubilisation in pancreatic steatorrhoea due to cystic fibrosis. Gut. 1985 Sep;26(9):892–901. doi: 10.1136/gut.26.9.892. [DOI] [PMC free article] [PubMed] [Google Scholar]
  55. Zentler-Munro P. L., Fine D. R., Fitzpatrick W. J., Northfield T. C. Effect of intrajejunal acidity on lipid digestion and aqueous solubilisation of bile acids and lipids in health, using a new simple method of lipase inactivation. Gut. 1984 May;25(5):491–499. doi: 10.1136/gut.25.5.491. [DOI] [PMC free article] [PubMed] [Google Scholar]
  56. Zerega J., Lerner S., Meyer J. H. Duodenal instillation of pancreatin does not abolish steatorrhea in patients with pancreatic insufficiency. Dig Dis Sci. 1988 Oct;33(10):1245–1249. doi: 10.1007/BF01536674. [DOI] [PubMed] [Google Scholar]

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